Mitochondria, the main provider of energy in eukaryotic cells, contains more than 1000
different proteins and is closely related to the development of cells. However, damaged …

Human caseinolytic protease P (ClpP) is required for the regulatory hydrolysis of
mitochondrial proteins. Allosteric ClpP agonists dysfunctionally activate mitochondrial ClpP …

Cancer cells alter the expression levels of metabolic enzymes to fuel proliferation. The
mitochondrion is a central hub of metabolic reprogramming, where chaperones service …

Mitochondria are key tumor drivers, but their suitability as a therapeutic target is unknown.
Here, we report on the preclinical characterization of Gamitrinib (GA mit ochondrial ma tri x …

Mitochondria are essential organelles in the human body, serving as the metabolic factory of
the whole organism. When mitochondria are dysfunctional, it can affect all organs of the …

The mitochondrial protein LonP1 is an ATP-dependent protease that mitigates cell stress
and calibrates mitochondrial metabolism and energetics. Biallelic mutations in the LONP1 …

Based on a clinically staged small molecular hClpP activator ONC201, a class of imipridone
derivatives was designed and synthesized. These compounds were evaluated in a protease …

Diffuse intrinsic pontine glioma (DIPG), affecting children aged 4–7 years, is a rare,
aggressive tumor that originates in the pons and then spreads to nearby tissue. DIPG is the …

Introduction Loss of proteasome function, proteinopathy, and proteotoxicity may cause
neurodegeneration across the human lifespan in several forms of brain injury and disease …

Friedreich's ataxia (FRDA) is a progressive neurodegenerative disease caused in almost all
patients by expanded guanine–adenine–adenine (GAA) trinucleotide repeats within intron 1 …